Skip to Main Content

++

  • Lou Gehrig’s disease
  • ALS
  • Motor neuron disease

++

  • 728.2 Muscular wasting and disuse atrophy, not elsewhere classified

++

  • M62.50 Muscle wasting and atrophy, not elsewhere classified, unspecified site

++
++

Description

++

  • Quickly progressive, degenerative motor disorder caused by the loss of nerve cells in the anterior horn of the spinal cord and motor nuclei of the brain stem2
  • Loss of muscle strength and coordination

++

Essentials of Diagnosis

++

  • Rapid, progressive loss of motor function not attributable to internal or external injury
  • Diagnosis made by evidence of motor loss in multiple parts of the body, speed of degeneration, and ruling out other progressive neurologic disorders

++

General Considerations

++

  • Onset may be either in the extremities or in the bulbar region initially; eventually, all patients experience degeneration in both areas
  • Diagnosis requires evidence of denervation in at least three motor regions
  • Usually does not affect the senses

++

Demographics

++

  • Male-to-female ratio: 2:13
  • Most patients aged > 45 years, but can be much younger3
  • 10% familial (tend to have onset at younger age)3

++

Signs and Symptoms

++

  • Both upper motor neuron and lower motor neuron signs and symptoms
  • Limb onset ALS
    • One side will be involved initially and spread to both sides within months
    • Patients usually report unexplained tripping while walking, or difficulty with buttons and small objects
    • Muscle fasciculation evident
    • Spasticity remains mild
  • Bulbar onset ALS
    • Difficulty with speech production; voice changes, hoarseness
    • Difficulty with swallowing; gagging, choking easily
    • Difficulty with saliva management, drooling
  • Sensation is spared, may experience transient paresthesias due to position or nerve compression
  • Bowel and bladder control usually maintained
  • Respiratory compromise evident with both bulbar and limb onset

++

Functional Implications

++

  • Loss of independence in mobility, self-care, eating, breathing
  • Dependence on others

++

Possible Contributing Causes

++

  • Primarily idiopathic
  • Family member who has a hereditary form

++

Differential Diagnosis

++

  • Progressive spinal muscular atrophy
  • Progressive bulbar palsy
  • Primary lateral sclerosis
  • Parkinson’s disease
  • Muscular dystrophy
  • Multiple sclerosis
  • Cervical spondylosis
  • Inflammatory myopathy
  • Myasthenia gravis
  • Inclusion body myositis

++

Laboratory Tests

++

  • Serum creatine kinase may be moderate elevated
  • Muscle biopsy
  • Genetic test for family history of ALS
  • Blood tests to rule out other conditions

++

Imaging

++

++

Diagnostic Procedures

++

  • Electromyography (EMG): widespread fasciculation and fibrillation
  • Motor nerve conduction velocity test (NCV); if positive, demonstrates slight slowing with focal motor conduction block
  • Swallowing study
  • Breathing test for lung involvement

++

Medication

...

Want remote access to your institution's subscription?

Sign in to your MyAccess profile while you are actively authenticated on this site via your institution (you will be able to verify this by looking at the top right corner of the screen - if you see your institution's name, you are authenticated). Once logged in to your MyAccess profile, you will be able to access your institution's subscription for 90 days from any location. You must be logged in while authenticated at least once every 90 days to maintain this remote access.

Ok

About MyAccess

If your institution subscribes to this resource, and you don't have a MyAccess profile, please contact your library's reference desk for information on how to gain access to this resource from off-campus.

Subscription Options

AccessPhysiotherapy Full Site: One-Year Subscription

Connect to the full suite of AccessPhysiotherapy content and resources including interactive NPTE review, more than 500 videos, Anatomy & Physiology Revealed, 20+ leading textbooks, and more.

$595 USD
Buy Now

Pay Per View: Timed Access to all of AccessPhysiotherapy

24 Hour Subscription $34.95

Buy Now

48 Hour Subscription $54.95

Buy Now

Pop-up div Successfully Displayed

This div only appears when the trigger link is hovered over. Otherwise it is hidden from view.